• Archives of Plastic Surgery
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• v.47 no.3
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• pp.272-276
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• 2020

Anorectal malformation or imperforate anus is a congenital anomaly of rectum and anus. Mullerian duct anomalies are abnormal development of uterus, cervix, and vagina. Imperforate anus with double uterus is extremely rare and cannot explain by normal embryologic development. Moreover, guideline in treatment is inconclusive. We report an extremely rare case of a young adult female who presented with recurrent pelvic inflammatory disease caused by rectovaginal fistula in congenital imperforate anus and didelphys uterus, and successfully neoanal reconstruction with gracilis muscle flap. Aims for treatment are closed rectovaginal fistula, and anal sphincter reconstruction. To our best knowledge, the imperforate anus with double uterus is extremely rare anomaly. Furthermore, successfully anal sphincter reconstruction with functional gracilis muscle in the imperforate anus with double uterus has never been reported in English literature.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.202-206
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• 2023

Cesarean scar pregnancy (CSP) is a rare complication that occurs in less than 1% of ectopic pregnancies, and uterine didelphys is one of the rarest uterine forms. We report a successful laparoscopic excision and repair of CSP in a woman with uterine didelphys and a double vagina. A 34-year-old gravida one, para one woman with a history of low transverse cesarean section presented to our hospital with a suspected CSP. She was confirmed to have uterine didelphys with a double vagina during an infertility examination 7 years earlier. Magnetic resonance imaging showed a 2.5-cm gestational sac-like cystic lesion in the lower segment of the right uterus at the cesarean scar. We decided to perform a laparoscopic approach after informing the patient of the surgical procedure. The lower segment of the previous cesarean site was excised with monopolar diathermy to minimize bleeding. We identified the gestational sac in the lower segment of the right uterus, which was evacuated using spoon forceps. The myometrium and serosa of the uterus were sutured layer-by-layer using synthetic absorbable sutures. No remnant gestational tissue was visible on follow-up ultrasonography one month after the surgery. This laparoscopic approach to CSP in a woman with uterine didelphys is an effective and safe method of treatment. In women with uterine anomalies, it is important to confirm the exact location of the gestational sac by preoperative imaging for successful surgery.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.299-305
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• 2007

Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.124-127
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• 2019

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.12-16
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• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

• Clinical and Experimental Reproductive Medicine
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• v.13 no.2
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• pp.137-144
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• 1986

A reveiw of 85 patients with uterine anomalies was made in respect to the incidence, chief complaints, the reason of infertility, fetal wastage rate, pregnancy complications, fetal presentations and obstetric outcome after metroplasty from 1980 to 1985. The results were summarized as follows: 1. Incidence of uterine anomaly was 0.18% among all outpatients (85/48,240). 2. Of the 85 patients, there were 36 with bicornuate deformities (42.3%), 21 septate (24.7%), 18 uterus didelphys (21.2%), 8 arcuate (9.4%) and 2 patients with unicornuate anomalies (2.4%). 3. Uterine anomalies were diagnosed by hysterosalpingogram (54.1%), pelvic examination (14.2%) and other operative procedures. 4. Chief complaints were primary infertility (41.2%), secondary infertility (15.3%), repeated pregnancy loss (12.9%), antenatal care (11.8%) and menstural disturbance (10.6%), etc. 5. Twenty-nine patients with uterine anomalies had primary infertility. The cause of infertility was proved nonuterine in 26 cases and remained unknown in 3 cases. 6. The obstetric outcome of 104 pregnancies was spontaneous abortion in 51.0%, premature delivery in 11.50/0 and fetal loss in 57.7%. 7. Complications of 41 present pregnancies were threatened abortion (22%), premature rupture of membrane (12%) and premature labor (10%), etc. The frequency of abnormal presentation was 35.3% and 64.7% of deliveries was made by Cesarian section. 8. Metroplasty was performed in 13 patients who didn't have a baby because of repeated miscarriage and unknown cause of infertility. Subsequently 8 patients had 9 successful pregnancies: 6 patients had 7 healthy babies and 2 patients are now in pregnancy without any complications.